Cronkhite-Canada syndrome (CCS) is a non-inherited GI disease. It is caused by multiple polyps in digestive tract and different abnormalities in the skin and nail. Multiple benign polyps which are occurred in the intestine and stomach can lead to bleeding and malabsorption of the nutrients. The familial adenomatous polyposis and other polyposis syndromes are different from these polyps.
Epidemiology
CSS is a scarce disease. It makes it very difficult to know the proper prevalence rate in general population. It was 1st describes in 1955 and few 100 cases were found in the globe.
It affects the old people whose age is about 50 to 60 years. It is seen in both men and women. The cases of CSS are found in different areas. It is not limited to particular area or group.
The incidence and prevalence are not certain because of the limited cases and difficulty to diagnose the disease.
Anatomy
Pathophysiology
Autoimmune Dysfunction: Some studies have indicated that CSS is an autoimmune disease. Autoimmune disease leads to inflammation and damage to the tissue. The immune system targets the GI tract and skin and cause polyps and changes in the skin in CSS.
Genetic Factors: CSS is not a hereditary disease. Some studies have indicated that there were some genetic factors which contribute to the development of the disease. It is not clear that CSS can lead to the genetic mutation.
Infectious Triggers: Infectious agents are involved in the development of the CSS. Some studies have found that the bacterial or viral infections are the triggers for the dysfunction in the immune system. There is not particular infectious agent is linked with the CSS.
Abnormal Immune Response in Gut: The GI polyps seen in the CSS are the result of the abnormal immune response in the gut. The response of the immune system can lead to inflammation and changes the cell growth and the formation of the many polyps. It can lead to GI symptoms like abdominal pain and diarrhea.
Nutritional Deficiencies: Patient who has CSS may have malabsorption of the nutrients because of the involvement of GI tract. These can lead to severe deficiency in the nutrition and cause weight loss and the systemic symptoms in disease.
Etiology
CSS is an idiopathic condition. The causes are not clear. There are many factors which can be the contributors to the disease:
Autoimmune Dysfunction: CSS can be linked to an autoimmune disease as the attacks the tissue of the immune system of body. It can lead to inflammation in GI tract and development of polyps. The particular autoantibodies included and the triggers remain not clear.
Genetic Predisposition: CSS is not a hereditary disease. Some studies indicated that a genetic predisposition or susceptibility to disease. There is not particular genetic mutation which are linked to CSS. Some environmental factors play a role in the development of the disease.
Infectious Triggers: Some researchers have explored the possibility that infectious agents, such as viruses or bacteria, could be involved in triggering CCS. However, no specific infectious agent has been definitively linked to the syndrome.
Environmental Factors: Some studies have indicated that CSS and exposure to toxins and substances can be linked. There is not clear link found.
Immune System Dysfunction: Dysfunction of the immune response in the GI tract may lead to development of polyps and other symptoms.
Abnormal Cell Signaling: Abnormal signaling in the GI tract cells can lead to the formation of the polyps and CSS.
Genetics
Prognostic Factors
CSS is a complex disease. It can vary from person to person. Some people who have remission or have benefit from the treatment may have continuous challenge or complications.
Old people may have a severe course of disease because of the health issues or decreased physiological activity. The number and distribution of the GI polyps can be the prognosis. If the polyps is spreading and many, a high risk of complications like bleeding, malabsorption and obstruction may occur. The response to the treatment is main prognostic factor. Some patients who have CSS respond good to the treatment and can manage with the deficiency of nutrition. Some have poor benefit from the treatment may lead to severe results.
Malabsorption of the nutrients is common in CSS. the severity of the deficiency of the nutrition can affect prognosis, and lead to weight loss, weakness and dysfunction in the immune function. Complication like protein loss, GI bleeding, electrolyte imbalance can affect the prognosis. Proper diagnosis and management of these complications is necessary to improve the results.
Pre-existing condition may affect the prognosis of the CSS. Patients who have chronic disease may reduce the resilience to get over from the CSS. Regular follow up and treatment plan is necessary to change the CSS.
Clinical History
Non-specific symptoms:
Diarrhea
Abdominal pain
Weight loss
Loss of appetite
Weakness and fatigue
Hyperpigmentation
Hypertrichosis
Abnormalities in nail and skin
Taste changes
Edema
Anemia
Systemic symptoms
Peripheral edema
Muscle weakness
Fatigue
Joint Pain
Neuropathy
Cardiovascular symptoms
Immunological abnormalities
Electrolyte imbalance
Anemia
Hypoalbuminemia
Age Group:
Adults
Old people
Physical Examination
Skin Examination: The healthcare provider will assess the skin for the any sign of hyperpigmentation like dark patches specifically on palms of hands, soles of feet and mucous membrane. Hypertrichosis can lead to increased hair growth can be seen.
Nail Examination: The healthcare provider will assess the nail for any signs of nail ridges, splitting or loss of nail plate.
Gastrointestinal Examination: The healthcare provider will assess the GI tract through the abdominal palpation to check for any signs of tenderness, masses or enlarged organ. They also look for malnutrition like muscle wasting and peripheral edema.
Vital Signs: Blood pressure, heart rate, and temperature will be assessed to check he overall health status.
Body Weight: It is necessary to monitor the body weight to check for unintended weight loss.
Skin Lesions: Any skin lesions or abnormalities specifically linked to hyperpigmentation will be checked.
Neurological Examination: Neurological examination is performed to check for neuropathy or other neurological diseases.
Evaluation of Mucous Membranes: The oral mucosa will be assessed for any signs of inflammation or pigmentation.
Age group
Adults Older individuals
Associated comorbidity
Malnutrition and Nutritional Deficiencies
Anemia
Electrolyte imbalance
Fluid and electrolyte disturbances
Immunological abnormalities
Autoimmune disease
Associated activity
Acuity of presentation
The acuity of presentation of CSS can differ on the basis of patient and the involvement of GI tract. CSS is a chronic disease, and symptoms are increases over a time. They can be diagnosed after many months.
The symptoms can be nonspecific and similar to the GI diseases in starting like diarrhea, abdominal pain, weight loss, and loss of appetite. Other symptoms are like hyperpigmentation and abnormalities in nail and skin as the progression of the disease.
CSS can become acute disease, and symptoms can be worsen and it needs immediate treatment. Acute complications are like GI bleeding, electrolyte imbalance.
Differential Diagnoses
Familial Adenomatous Polyposis (FAP): FAP is a hereditary condition which is caused by the development of many adenomatous polyps in the colon and rectum. Polyps in the CSS are non-adenomatous and overlapping which makes it differ from others.
Peutz-Jeghers Syndrome: Peutz-Jeghers syndrome id genetic disease which is caused by polyps in the GI tract. Patient who has this disease may have pigmented spots on lips, buccal mucosa, and hands. The pigmentation in the CSS are wide spread and included on the palms and soles.
Inflammatory Bowel Disease (IBD): Conditions like Crohn’s disease and ulcerative colitis are forms of IBD which has symptoms like chronic diarrhea, abdominal pain and weight gain similar to CSS. CSS does not have inflammatory changes which have been seen in IBD on biopsy and endoscopy.
Gastric Cancer: Gastric cancer can be seen with polyps in stomach same as CSS. It is necessary to perform proper imaging and biopsy to exclude it.
Gastrointestinal Stromal Tumors (GISTs): GISTs are rare tumors which can occur in the GI tract and may be mistaken for polyps in the CCS. Imaging and biopsy are necessary to diagnose.
Malabsorption Syndromes: Conditions which can lead to malabsorption of nutrients like celiac disease or tropical sprue may have symptoms like the CCS. Laboratory tests and small bowel biopsy can be useful to differentiate the disease.
Autoimmune Enteropathy: This rare autoimmune disorder which can cause severe malabsorption and diarrhea have same symptoms like CSS.
Infectious Gastroenteritis: Acute or chronic infectious gastroenteritis can have symptoms like diarrhea and abdominal pain same as CCS. A history and stool studies are needed to identify the infectious causes.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Nutritional Support: CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals. Take advise from the dietitian or nutritionist to make a diet plan.
Medications: Medications may be prescribed to control symptoms like diarrhea, abdominal pain, and inflammation. Anti-diarrheal drugs and anti-inflammatory agents is used to manage the disease.
Immunosuppressive Therapy: Immunosuppressive medications can be used to increase the immune response.
Endoscopic Polypectomy: It is necessary to remove large or problematic GI polyps by endoscopy to reduce the symptoms or prevent complications like bleeding or obstruction.
Corticosteroids: Corticosteroids like prednisone is used to improve the symptoms. The long-term usage is linked with side effects.
Immunomodulatory Therapies: Other immunomodulatory agents like infliximab or cyclosporine are used to treat CSS but the usage is limited.
Regular Monitoring: Take follow-up and monitoring is necessary to check the treatment response and status of nutrition and complications.
Supportive Care: Give emotional and psychological support is needed for patient who has CSS.
Management of Complications: Complications like GI bleeding, fluid and electrolyte imbalances, or infections can occur in the CSS. It needs immediate management and treatment.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
monitor-the-patient
Monitoring and Surveillance: Regular monitoring is necessary to check the progression of the CSS, to manage the symptoms and detect the complications. Monitor the patient who have CSS and high risk of GI cancer.
Psychosocial Support: CCS can affect the physical and emotional health of patient. Psychosocial support, counseling and care from support group is necessary to manage the diseae.
Nutritional Support: CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals.
Treatment of Symptoms: Particular symptoms like diarrhea, abdominal pain and weight loss can be treated with medications.
Administration of corticosteroids to treat Cronkhite-Canada Syndrome
Prednisone: It is a corticosteroid and anti-inflammatory drug. It reduces the abnormal function of immune system. It reduces inflammation and symptoms and improves the overall health of patient who has CSS.
It is administered orally. The dosage and duration may vary on the basis of the severity of disease and response to the treatment. Close monitoring is necessary to check the effectiveness and for the adjustment of the dosage.
Administration of antibiotics to treat Cronkhite-Canada Syndrome:
Tetracycline: It has anti-inflammatory effects. It modulates the immune response and manage the CSS. It is used along with corticosteroids or immunosuppressive medications as an adjunctive therapy. It is given on the basis of the condition, symptoms, and response to treatment of patient.
Metronidazole: It has anti-inflammatory property. It is used in GI diseases because of the effects of gut flora and immune modulation.
Administration of TNF inhibitors for the treatment of Cronkhite-Canada Syndrome
Infliximab: It is used to target the inflammatory process and dysregulation of immune response. It inhibits the TNF-α and reduces the inflammation and improve the GI symptoms.
Endoscopy: Endoscopy allows to see the mucosal lining and polyps in the esophagus, stomach, small intestine, and colon. It is used to diagnose CSS, assess the polyp burden and to collect the biopsy samples for histological examinations.
Polypectomy: Polypectomy is performed during endoscopy to remove the GI polyps. The polyps may lead to bleeding, obstruction, or other complications. Polypectomy can reduce these complications.
Nutritional Support: Enteral or parenteral nutrition can be given to patient who has CSS. Enteral nutrition is given by a feeding tube directly to the GI tract. Parenteral nutrition is given by IV when GI tract cannot absorb the nutrient properly.
Surgical Interventions: Surgical procedures are needed to remove the complications or reduce the severe symptoms linked with the CSS. Surgery can be performed to manage the GI bleeding, remove the large polyps or tumor or to manage intestinal obstruction.
the-phase-of-management
Diagnostic Phase:
It is necessary to identify the main cause of the CSS in starting phase. It includes the evaluation like medical history, physical examination and laboratory test. Diagnostic tests like endoscopy and biopsy can conform the presence of GI polyps and imaging study is used to know the progression of polyps. Other diseases with same symptoms must be excluded during this phase.
Acute Symptomatic Management Phase:
If patient has severe or acute symptoms like diarrhea, abdominal pain, and malnutrition medication is administered to reduce the symptoms. Supportive care like fluid and electrolyte management is needed in patient who has losses of fluid and electrolytes.
Nutritional Support Phase:
CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals. Monitor the eight and nutritional status of patient is necessary during this phase.
Maintenance Phase:
The Patient will enter into the maintenance phase after controlling the symptoms and nutritional deficiency. It needs medical monitoring to check the treatment response, complication and adjust the dosage if needed.
Long-Term Management Phase:
Chronic CCS needs long-term management to control the symptoms and prevent complications. The treatment plan needs to be adjusted on the basis of response of patient to treatment and changes in the symptoms or nutritional status.
Complications and Relapse Management Phase:
Immediate management of complications like gastrointestinal bleeding and electrolyte imbalances is important to prevent deterioration. Re-evaluation of treatment plan and interventions is needed.
Cronkhite-Canada syndrome (CCS) is a non-inherited GI disease. It is caused by multiple polyps in digestive tract and different abnormalities in the skin and nail. Multiple benign polyps which are occurred in the intestine and stomach can lead to bleeding and malabsorption of the nutrients. The familial adenomatous polyposis and other polyposis syndromes are different from these polyps.
CSS is a scarce disease. It makes it very difficult to know the proper prevalence rate in general population. It was 1st describes in 1955 and few 100 cases were found in the globe.
It affects the old people whose age is about 50 to 60 years. It is seen in both men and women. The cases of CSS are found in different areas. It is not limited to particular area or group.
The incidence and prevalence are not certain because of the limited cases and difficulty to diagnose the disease.
Autoimmune Dysfunction: Some studies have indicated that CSS is an autoimmune disease. Autoimmune disease leads to inflammation and damage to the tissue. The immune system targets the GI tract and skin and cause polyps and changes in the skin in CSS.
Genetic Factors: CSS is not a hereditary disease. Some studies have indicated that there were some genetic factors which contribute to the development of the disease. It is not clear that CSS can lead to the genetic mutation.
Infectious Triggers: Infectious agents are involved in the development of the CSS. Some studies have found that the bacterial or viral infections are the triggers for the dysfunction in the immune system. There is not particular infectious agent is linked with the CSS.
Abnormal Immune Response in Gut: The GI polyps seen in the CSS are the result of the abnormal immune response in the gut. The response of the immune system can lead to inflammation and changes the cell growth and the formation of the many polyps. It can lead to GI symptoms like abdominal pain and diarrhea.
Nutritional Deficiencies: Patient who has CSS may have malabsorption of the nutrients because of the involvement of GI tract. These can lead to severe deficiency in the nutrition and cause weight loss and the systemic symptoms in disease.
CSS is an idiopathic condition. The causes are not clear. There are many factors which can be the contributors to the disease:
Autoimmune Dysfunction: CSS can be linked to an autoimmune disease as the attacks the tissue of the immune system of body. It can lead to inflammation in GI tract and development of polyps. The particular autoantibodies included and the triggers remain not clear.
Genetic Predisposition: CSS is not a hereditary disease. Some studies indicated that a genetic predisposition or susceptibility to disease. There is not particular genetic mutation which are linked to CSS. Some environmental factors play a role in the development of the disease.
Infectious Triggers: Some researchers have explored the possibility that infectious agents, such as viruses or bacteria, could be involved in triggering CCS. However, no specific infectious agent has been definitively linked to the syndrome.
Environmental Factors: Some studies have indicated that CSS and exposure to toxins and substances can be linked. There is not clear link found.
Immune System Dysfunction: Dysfunction of the immune response in the GI tract may lead to development of polyps and other symptoms.
Abnormal Cell Signaling: Abnormal signaling in the GI tract cells can lead to the formation of the polyps and CSS.
CSS is a complex disease. It can vary from person to person. Some people who have remission or have benefit from the treatment may have continuous challenge or complications.
Old people may have a severe course of disease because of the health issues or decreased physiological activity. The number and distribution of the GI polyps can be the prognosis. If the polyps is spreading and many, a high risk of complications like bleeding, malabsorption and obstruction may occur. The response to the treatment is main prognostic factor. Some patients who have CSS respond good to the treatment and can manage with the deficiency of nutrition. Some have poor benefit from the treatment may lead to severe results.
Malabsorption of the nutrients is common in CSS. the severity of the deficiency of the nutrition can affect prognosis, and lead to weight loss, weakness and dysfunction in the immune function. Complication like protein loss, GI bleeding, electrolyte imbalance can affect the prognosis. Proper diagnosis and management of these complications is necessary to improve the results.
Pre-existing condition may affect the prognosis of the CSS. Patients who have chronic disease may reduce the resilience to get over from the CSS. Regular follow up and treatment plan is necessary to change the CSS.
Non-specific symptoms:
Diarrhea
Abdominal pain
Weight loss
Loss of appetite
Weakness and fatigue
Hyperpigmentation
Hypertrichosis
Abnormalities in nail and skin
Taste changes
Edema
Anemia
Systemic symptoms
Peripheral edema
Muscle weakness
Fatigue
Joint Pain
Neuropathy
Cardiovascular symptoms
Immunological abnormalities
Electrolyte imbalance
Anemia
Hypoalbuminemia
Age Group:
Adults
Old people
Skin Examination: The healthcare provider will assess the skin for the any sign of hyperpigmentation like dark patches specifically on palms of hands, soles of feet and mucous membrane. Hypertrichosis can lead to increased hair growth can be seen.
Nail Examination: The healthcare provider will assess the nail for any signs of nail ridges, splitting or loss of nail plate.
Gastrointestinal Examination: The healthcare provider will assess the GI tract through the abdominal palpation to check for any signs of tenderness, masses or enlarged organ. They also look for malnutrition like muscle wasting and peripheral edema.
Vital Signs: Blood pressure, heart rate, and temperature will be assessed to check he overall health status.
Body Weight: It is necessary to monitor the body weight to check for unintended weight loss.
Skin Lesions: Any skin lesions or abnormalities specifically linked to hyperpigmentation will be checked.
Neurological Examination: Neurological examination is performed to check for neuropathy or other neurological diseases.
Evaluation of Mucous Membranes: The oral mucosa will be assessed for any signs of inflammation or pigmentation.
Adults Older individuals
Malnutrition and Nutritional Deficiencies
Anemia
Electrolyte imbalance
Fluid and electrolyte disturbances
Immunological abnormalities
Autoimmune disease
The acuity of presentation of CSS can differ on the basis of patient and the involvement of GI tract. CSS is a chronic disease, and symptoms are increases over a time. They can be diagnosed after many months.
The symptoms can be nonspecific and similar to the GI diseases in starting like diarrhea, abdominal pain, weight loss, and loss of appetite. Other symptoms are like hyperpigmentation and abnormalities in nail and skin as the progression of the disease.
CSS can become acute disease, and symptoms can be worsen and it needs immediate treatment. Acute complications are like GI bleeding, electrolyte imbalance.
Familial Adenomatous Polyposis (FAP): FAP is a hereditary condition which is caused by the development of many adenomatous polyps in the colon and rectum. Polyps in the CSS are non-adenomatous and overlapping which makes it differ from others.
Peutz-Jeghers Syndrome: Peutz-Jeghers syndrome id genetic disease which is caused by polyps in the GI tract. Patient who has this disease may have pigmented spots on lips, buccal mucosa, and hands. The pigmentation in the CSS are wide spread and included on the palms and soles.
Inflammatory Bowel Disease (IBD): Conditions like Crohn’s disease and ulcerative colitis are forms of IBD which has symptoms like chronic diarrhea, abdominal pain and weight gain similar to CSS. CSS does not have inflammatory changes which have been seen in IBD on biopsy and endoscopy.
Gastric Cancer: Gastric cancer can be seen with polyps in stomach same as CSS. It is necessary to perform proper imaging and biopsy to exclude it.
Gastrointestinal Stromal Tumors (GISTs): GISTs are rare tumors which can occur in the GI tract and may be mistaken for polyps in the CCS. Imaging and biopsy are necessary to diagnose.
Malabsorption Syndromes: Conditions which can lead to malabsorption of nutrients like celiac disease or tropical sprue may have symptoms like the CCS. Laboratory tests and small bowel biopsy can be useful to differentiate the disease.
Autoimmune Enteropathy: This rare autoimmune disorder which can cause severe malabsorption and diarrhea have same symptoms like CSS.
Infectious Gastroenteritis: Acute or chronic infectious gastroenteritis can have symptoms like diarrhea and abdominal pain same as CCS. A history and stool studies are needed to identify the infectious causes.
Nutritional Support: CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals. Take advise from the dietitian or nutritionist to make a diet plan.
Medications: Medications may be prescribed to control symptoms like diarrhea, abdominal pain, and inflammation. Anti-diarrheal drugs and anti-inflammatory agents is used to manage the disease.
Immunosuppressive Therapy: Immunosuppressive medications can be used to increase the immune response.
Endoscopic Polypectomy: It is necessary to remove large or problematic GI polyps by endoscopy to reduce the symptoms or prevent complications like bleeding or obstruction.
Corticosteroids: Corticosteroids like prednisone is used to improve the symptoms. The long-term usage is linked with side effects.
Immunomodulatory Therapies: Other immunomodulatory agents like infliximab or cyclosporine are used to treat CSS but the usage is limited.
Regular Monitoring: Take follow-up and monitoring is necessary to check the treatment response and status of nutrition and complications.
Supportive Care: Give emotional and psychological support is needed for patient who has CSS.
Management of Complications: Complications like GI bleeding, fluid and electrolyte imbalances, or infections can occur in the CSS. It needs immediate management and treatment.
Dermatology, General
Gastroenterology
Nutrition
Monitoring and Surveillance: Regular monitoring is necessary to check the progression of the CSS, to manage the symptoms and detect the complications. Monitor the patient who have CSS and high risk of GI cancer.
Psychosocial Support: CCS can affect the physical and emotional health of patient. Psychosocial support, counseling and care from support group is necessary to manage the diseae.
Dermatology, General
Gastroenterology
Nutrition
Nutritional Support: CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals.
Treatment of Symptoms: Particular symptoms like diarrhea, abdominal pain and weight loss can be treated with medications.
Dermatology, General
Nutrition
Rheumatology
Prednisone: It is a corticosteroid and anti-inflammatory drug. It reduces the abnormal function of immune system. It reduces inflammation and symptoms and improves the overall health of patient who has CSS.
It is administered orally. The dosage and duration may vary on the basis of the severity of disease and response to the treatment. Close monitoring is necessary to check the effectiveness and for the adjustment of the dosage.
Dermatology, General
Rheumatology
Tetracycline: It has anti-inflammatory effects. It modulates the immune response and manage the CSS. It is used along with corticosteroids or immunosuppressive medications as an adjunctive therapy. It is given on the basis of the condition, symptoms, and response to treatment of patient.
Metronidazole: It has anti-inflammatory property. It is used in GI diseases because of the effects of gut flora and immune modulation.
Dermatology, General
Infliximab: It is used to target the inflammatory process and dysregulation of immune response. It inhibits the TNF-α and reduces the inflammation and improve the GI symptoms.
Dermatology, General
Diagnostic Phase:
It is necessary to identify the main cause of the CSS in starting phase. It includes the evaluation like medical history, physical examination and laboratory test. Diagnostic tests like endoscopy and biopsy can conform the presence of GI polyps and imaging study is used to know the progression of polyps. Other diseases with same symptoms must be excluded during this phase.
Acute Symptomatic Management Phase:
If patient has severe or acute symptoms like diarrhea, abdominal pain, and malnutrition medication is administered to reduce the symptoms. Supportive care like fluid and electrolyte management is needed in patient who has losses of fluid and electrolytes.
Nutritional Support Phase:
CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals. Monitor the eight and nutritional status of patient is necessary during this phase.
Maintenance Phase:
The Patient will enter into the maintenance phase after controlling the symptoms and nutritional deficiency. It needs medical monitoring to check the treatment response, complication and adjust the dosage if needed.
Long-Term Management Phase:
Chronic CCS needs long-term management to control the symptoms and prevent complications. The treatment plan needs to be adjusted on the basis of response of patient to treatment and changes in the symptoms or nutritional status.
Complications and Relapse Management Phase:
Immediate management of complications like gastrointestinal bleeding and electrolyte imbalances is important to prevent deterioration. Re-evaluation of treatment plan and interventions is needed.
Cronkhite-Canada syndrome (CCS) is a non-inherited GI disease. It is caused by multiple polyps in digestive tract and different abnormalities in the skin and nail. Multiple benign polyps which are occurred in the intestine and stomach can lead to bleeding and malabsorption of the nutrients. The familial adenomatous polyposis and other polyposis syndromes are different from these polyps.
CSS is a scarce disease. It makes it very difficult to know the proper prevalence rate in general population. It was 1st describes in 1955 and few 100 cases were found in the globe.
It affects the old people whose age is about 50 to 60 years. It is seen in both men and women. The cases of CSS are found in different areas. It is not limited to particular area or group.
The incidence and prevalence are not certain because of the limited cases and difficulty to diagnose the disease.
Autoimmune Dysfunction: Some studies have indicated that CSS is an autoimmune disease. Autoimmune disease leads to inflammation and damage to the tissue. The immune system targets the GI tract and skin and cause polyps and changes in the skin in CSS.
Genetic Factors: CSS is not a hereditary disease. Some studies have indicated that there were some genetic factors which contribute to the development of the disease. It is not clear that CSS can lead to the genetic mutation.
Infectious Triggers: Infectious agents are involved in the development of the CSS. Some studies have found that the bacterial or viral infections are the triggers for the dysfunction in the immune system. There is not particular infectious agent is linked with the CSS.
Abnormal Immune Response in Gut: The GI polyps seen in the CSS are the result of the abnormal immune response in the gut. The response of the immune system can lead to inflammation and changes the cell growth and the formation of the many polyps. It can lead to GI symptoms like abdominal pain and diarrhea.
Nutritional Deficiencies: Patient who has CSS may have malabsorption of the nutrients because of the involvement of GI tract. These can lead to severe deficiency in the nutrition and cause weight loss and the systemic symptoms in disease.
CSS is an idiopathic condition. The causes are not clear. There are many factors which can be the contributors to the disease:
Autoimmune Dysfunction: CSS can be linked to an autoimmune disease as the attacks the tissue of the immune system of body. It can lead to inflammation in GI tract and development of polyps. The particular autoantibodies included and the triggers remain not clear.
Genetic Predisposition: CSS is not a hereditary disease. Some studies indicated that a genetic predisposition or susceptibility to disease. There is not particular genetic mutation which are linked to CSS. Some environmental factors play a role in the development of the disease.
Infectious Triggers: Some researchers have explored the possibility that infectious agents, such as viruses or bacteria, could be involved in triggering CCS. However, no specific infectious agent has been definitively linked to the syndrome.
Environmental Factors: Some studies have indicated that CSS and exposure to toxins and substances can be linked. There is not clear link found.
Immune System Dysfunction: Dysfunction of the immune response in the GI tract may lead to development of polyps and other symptoms.
Abnormal Cell Signaling: Abnormal signaling in the GI tract cells can lead to the formation of the polyps and CSS.
CSS is a complex disease. It can vary from person to person. Some people who have remission or have benefit from the treatment may have continuous challenge or complications.
Old people may have a severe course of disease because of the health issues or decreased physiological activity. The number and distribution of the GI polyps can be the prognosis. If the polyps is spreading and many, a high risk of complications like bleeding, malabsorption and obstruction may occur. The response to the treatment is main prognostic factor. Some patients who have CSS respond good to the treatment and can manage with the deficiency of nutrition. Some have poor benefit from the treatment may lead to severe results.
Malabsorption of the nutrients is common in CSS. the severity of the deficiency of the nutrition can affect prognosis, and lead to weight loss, weakness and dysfunction in the immune function. Complication like protein loss, GI bleeding, electrolyte imbalance can affect the prognosis. Proper diagnosis and management of these complications is necessary to improve the results.
Pre-existing condition may affect the prognosis of the CSS. Patients who have chronic disease may reduce the resilience to get over from the CSS. Regular follow up and treatment plan is necessary to change the CSS.
Non-specific symptoms:
Diarrhea
Abdominal pain
Weight loss
Loss of appetite
Weakness and fatigue
Hyperpigmentation
Hypertrichosis
Abnormalities in nail and skin
Taste changes
Edema
Anemia
Systemic symptoms
Peripheral edema
Muscle weakness
Fatigue
Joint Pain
Neuropathy
Cardiovascular symptoms
Immunological abnormalities
Electrolyte imbalance
Anemia
Hypoalbuminemia
Age Group:
Adults
Old people
Skin Examination: The healthcare provider will assess the skin for the any sign of hyperpigmentation like dark patches specifically on palms of hands, soles of feet and mucous membrane. Hypertrichosis can lead to increased hair growth can be seen.
Nail Examination: The healthcare provider will assess the nail for any signs of nail ridges, splitting or loss of nail plate.
Gastrointestinal Examination: The healthcare provider will assess the GI tract through the abdominal palpation to check for any signs of tenderness, masses or enlarged organ. They also look for malnutrition like muscle wasting and peripheral edema.
Vital Signs: Blood pressure, heart rate, and temperature will be assessed to check he overall health status.
Body Weight: It is necessary to monitor the body weight to check for unintended weight loss.
Skin Lesions: Any skin lesions or abnormalities specifically linked to hyperpigmentation will be checked.
Neurological Examination: Neurological examination is performed to check for neuropathy or other neurological diseases.
Evaluation of Mucous Membranes: The oral mucosa will be assessed for any signs of inflammation or pigmentation.
Adults Older individuals
Malnutrition and Nutritional Deficiencies
Anemia
Electrolyte imbalance
Fluid and electrolyte disturbances
Immunological abnormalities
Autoimmune disease
The acuity of presentation of CSS can differ on the basis of patient and the involvement of GI tract. CSS is a chronic disease, and symptoms are increases over a time. They can be diagnosed after many months.
The symptoms can be nonspecific and similar to the GI diseases in starting like diarrhea, abdominal pain, weight loss, and loss of appetite. Other symptoms are like hyperpigmentation and abnormalities in nail and skin as the progression of the disease.
CSS can become acute disease, and symptoms can be worsen and it needs immediate treatment. Acute complications are like GI bleeding, electrolyte imbalance.
Familial Adenomatous Polyposis (FAP): FAP is a hereditary condition which is caused by the development of many adenomatous polyps in the colon and rectum. Polyps in the CSS are non-adenomatous and overlapping which makes it differ from others.
Peutz-Jeghers Syndrome: Peutz-Jeghers syndrome id genetic disease which is caused by polyps in the GI tract. Patient who has this disease may have pigmented spots on lips, buccal mucosa, and hands. The pigmentation in the CSS are wide spread and included on the palms and soles.
Inflammatory Bowel Disease (IBD): Conditions like Crohn’s disease and ulcerative colitis are forms of IBD which has symptoms like chronic diarrhea, abdominal pain and weight gain similar to CSS. CSS does not have inflammatory changes which have been seen in IBD on biopsy and endoscopy.
Gastric Cancer: Gastric cancer can be seen with polyps in stomach same as CSS. It is necessary to perform proper imaging and biopsy to exclude it.
Gastrointestinal Stromal Tumors (GISTs): GISTs are rare tumors which can occur in the GI tract and may be mistaken for polyps in the CCS. Imaging and biopsy are necessary to diagnose.
Malabsorption Syndromes: Conditions which can lead to malabsorption of nutrients like celiac disease or tropical sprue may have symptoms like the CCS. Laboratory tests and small bowel biopsy can be useful to differentiate the disease.
Autoimmune Enteropathy: This rare autoimmune disorder which can cause severe malabsorption and diarrhea have same symptoms like CSS.
Infectious Gastroenteritis: Acute or chronic infectious gastroenteritis can have symptoms like diarrhea and abdominal pain same as CCS. A history and stool studies are needed to identify the infectious causes.
Nutritional Support: CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals. Take advise from the dietitian or nutritionist to make a diet plan.
Medications: Medications may be prescribed to control symptoms like diarrhea, abdominal pain, and inflammation. Anti-diarrheal drugs and anti-inflammatory agents is used to manage the disease.
Immunosuppressive Therapy: Immunosuppressive medications can be used to increase the immune response.
Endoscopic Polypectomy: It is necessary to remove large or problematic GI polyps by endoscopy to reduce the symptoms or prevent complications like bleeding or obstruction.
Corticosteroids: Corticosteroids like prednisone is used to improve the symptoms. The long-term usage is linked with side effects.
Immunomodulatory Therapies: Other immunomodulatory agents like infliximab or cyclosporine are used to treat CSS but the usage is limited.
Regular Monitoring: Take follow-up and monitoring is necessary to check the treatment response and status of nutrition and complications.
Supportive Care: Give emotional and psychological support is needed for patient who has CSS.
Management of Complications: Complications like GI bleeding, fluid and electrolyte imbalances, or infections can occur in the CSS. It needs immediate management and treatment.
Dermatology, General
Gastroenterology
Nutrition
Monitoring and Surveillance: Regular monitoring is necessary to check the progression of the CSS, to manage the symptoms and detect the complications. Monitor the patient who have CSS and high risk of GI cancer.
Psychosocial Support: CCS can affect the physical and emotional health of patient. Psychosocial support, counseling and care from support group is necessary to manage the diseae.
Dermatology, General
Gastroenterology
Nutrition
Nutritional Support: CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals.
Treatment of Symptoms: Particular symptoms like diarrhea, abdominal pain and weight loss can be treated with medications.
Dermatology, General
Nutrition
Rheumatology
Prednisone: It is a corticosteroid and anti-inflammatory drug. It reduces the abnormal function of immune system. It reduces inflammation and symptoms and improves the overall health of patient who has CSS.
It is administered orally. The dosage and duration may vary on the basis of the severity of disease and response to the treatment. Close monitoring is necessary to check the effectiveness and for the adjustment of the dosage.
Dermatology, General
Rheumatology
Tetracycline: It has anti-inflammatory effects. It modulates the immune response and manage the CSS. It is used along with corticosteroids or immunosuppressive medications as an adjunctive therapy. It is given on the basis of the condition, symptoms, and response to treatment of patient.
Metronidazole: It has anti-inflammatory property. It is used in GI diseases because of the effects of gut flora and immune modulation.
Dermatology, General
Infliximab: It is used to target the inflammatory process and dysregulation of immune response. It inhibits the TNF-α and reduces the inflammation and improve the GI symptoms.
Dermatology, General
Endoscopy: Endoscopy allows to see the mucosal lining and polyps in the esophagus, stomach, small intestine, and colon. It is used to diagnose CSS, assess the polyp burden and to collect the biopsy samples for histological examinations.
Polypectomy: Polypectomy is performed during endoscopy to remove the GI polyps. The polyps may lead to bleeding, obstruction, or other complications. Polypectomy can reduce these complications.
Nutritional Support: Enteral or parenteral nutrition can be given to patient who has CSS. Enteral nutrition is given by a feeding tube directly to the GI tract. Parenteral nutrition is given by IV when GI tract cannot absorb the nutrient properly.
Surgical Interventions: Surgical procedures are needed to remove the complications or reduce the severe symptoms linked with the CSS. Surgery can be performed to manage the GI bleeding, remove the large polyps or tumor or to manage intestinal obstruction.
Dermatology, General
Diagnostic Phase:
It is necessary to identify the main cause of the CSS in starting phase. It includes the evaluation like medical history, physical examination and laboratory test. Diagnostic tests like endoscopy and biopsy can conform the presence of GI polyps and imaging study is used to know the progression of polyps. Other diseases with same symptoms must be excluded during this phase.
Acute Symptomatic Management Phase:
If patient has severe or acute symptoms like diarrhea, abdominal pain, and malnutrition medication is administered to reduce the symptoms. Supportive care like fluid and electrolyte management is needed in patient who has losses of fluid and electrolytes.
Nutritional Support Phase:
CSS can lead to malabsorption of the nutrition. Nutrition support is necessary to manage the disease. It can include modification in the diet and supplement like iron, vitamins, and minerals. Monitor the eight and nutritional status of patient is necessary during this phase.
Maintenance Phase:
The Patient will enter into the maintenance phase after controlling the symptoms and nutritional deficiency. It needs medical monitoring to check the treatment response, complication and adjust the dosage if needed.
Long-Term Management Phase:
Chronic CCS needs long-term management to control the symptoms and prevent complications. The treatment plan needs to be adjusted on the basis of response of patient to treatment and changes in the symptoms or nutritional status.
Complications and Relapse Management Phase:
Immediate management of complications like gastrointestinal bleeding and electrolyte imbalances is important to prevent deterioration. Re-evaluation of treatment plan and interventions is needed.
Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.
Digital Certificate PDF
On course completion, you will receive a full-sized presentation quality digital certificate.
medtigo Simulation
A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.
medtigo Points
medtigo points is our unique point redemption system created to award users for interacting on our site. These points can be redeemed for special discounts on the medtigo marketplace as well as towards the membership cost itself.
Community Forum post/reply = 5 points
*Redemption of points can occur only through the medtigo marketplace, courses, or simulation system. Money will not be credited to your bank account. 10 points = $1.
All Your Certificates in One Place
When you have your licenses, certificates and CMEs in one place, it's easier to track your career growth. You can easily share these with hospitals as well, using your medtigo app.
